A Novel KIDINS220 Pathogenic Variant Associated with the Syndromic Spastic Paraplegia SINO: An Expansion of the Brain Malformation Spectrum and a Literature Review.

axon guidance defects macrocephaly obesity scaffold protein spastic paraplegia tubulin-related disorders

Journal

Genes
ISSN: 2073-4425
Titre abrégé: Genes (Basel)
Pays: Switzerland
ID NLM: 101551097

Informations de publication

Date de publication:
10 Sep 2024
Historique:
received: 21 08 2024
revised: 30 08 2024
accepted: 30 08 2024
medline: 29 9 2024
pubmed: 28 9 2024
entrez: 28 9 2024
Statut: epublish

Résumé

Identifying novel variants in very rare disease genes can be challenging when patients exhibit a complex phenotype that expands the one described, and we provide such an example here. A few terminal truncating variants in We performed whole exome sequencing (WES) and a literature and mutation databases review. We report a young adult with SP, severe ID, strabismus, and macrocephaly exhibiting brain malformations at follow-up, partially overlapping with those described in The progression of myelination and the development of brain structures turned out to be crucial for identifying, at follow-up, the whole

Sections du résumé

BACKGROUND/OBJECTIVES OBJECTIVE
Identifying novel variants in very rare disease genes can be challenging when patients exhibit a complex phenotype that expands the one described, and we provide such an example here. A few terminal truncating variants in
METHODS METHODS
We performed whole exome sequencing (WES) and a literature and mutation databases review.
RESULTS RESULTS
We report a young adult with SP, severe ID, strabismus, and macrocephaly exhibiting brain malformations at follow-up, partially overlapping with those described in
CONCLUSIONS CONCLUSIONS
The progression of myelination and the development of brain structures turned out to be crucial for identifying, at follow-up, the whole

Identifiants

pubmed: 39336781
pii: genes15091190
doi: 10.3390/genes15091190
pii:
doi:

Types de publication

Journal Article Case Reports Review

Langues

eng

Sous-ensembles de citation

IM

Auteurs

Maria Teresa Bonati (MT)

Institute for Maternal and Child Health "Burlo Garofolo", 34137 Trieste, TS, Italy.

Cristina Baldoli (C)

Department of Neuroradiology, San Raffaele Scientific Institute, 20132 Milan, MI, Italy.

Jacopo Taurino (J)

Cardiovascular-Genetic Center, IRCCS Policlinico San Donato, 20097 San Donato Milanese, MI, Italy.

Daniela Marchetti (D)

Laboratorio di Genetica Medica, Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII, 24127 Bergamo, BG, Italy.

Lidia Larizza (L)

Research Laboratory of Medical Cytogenetics and Molecular Genetics, IRCCS Istituto Auxologico Italiano, 20095 Cusano Milanino, MI, Italy.

Palma Finelli (P)

SC Patologia Clinica, SS Laboratorio Genetica Medica, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milano, MI, Italy.
Department of Medical Biotechnology and Translational Medicine, University of Milan, 20122 Milan, MI, Italy.

Maria Iascone (M)

Laboratorio di Genetica Medica, Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII, 24127 Bergamo, BG, Italy.

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Classifications MeSH